Rare Diseases Symptoms Automatic Extraction

Responsiveness of nonHerlitz junctional epidermolysis bullosa to topical gentian violet.

[junctional epidermolysis bullosa]

Gentian violet (GV), a mixture of crystal violet and methyl violet, a dye belonging to the di- and triaminophenylmethanes class and has been widely used for its bactericidal and fungicidal properties. To date, there have been no reports of long-term therapeutic use of GV in epidermolysis bullosa (EB).Two brothers with nonHerlitz junctional epidermolysis bullosa (JEB) aged 12 and 14 tried topical GV to one lower leg with conventional silicone dressings and this was compared with leaving the other leg with silicone dressings alone, over 4 weeks. Wounds were photographed and measured using Visitrak analysis. Pain, ooze, and appearance were assessed using visual analog scales (VAS) scales and Quality of life using Dermatology Life Quality Index and QOLEB (2) tools.The side treated with dressings and GV reduced to 14.9 cm(2) (-20.74%) and to 9.5 cm(2) (-56.62%) for dressings alone in the older brother (EB-012) and to 4.2 cm(2) (+20%) and 12.5 cm(2) (-7%) for the younger brother (EB-011) in ulcer size, respectively. Both patients did complain of stinging on the sites treated within a few days. QOL measures and VAS scores did not show any significant change.GV may be considered to be a therapeutic option for ulcers in nH-JEB patients and potentially other EB subtypes. A formal randomized controlled trial and long-term safety study of GV in EB is recommended.

Diseases presenting "leg with conventional silicone dressings" symptom

  • junctional epidermolysis bullosa

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